Burkitt's lymphoma
ℹ️ About
- 🧬 Monoclonal proliferation of B lymphocytes.
- ⚡ Very aggressive form of non-Hodgkin lymphoma (<1% of B-cell lymphomas).
- 👨⚕️ First described by Denis Burkitt, Irish surgeon working in Africa (1950s).
- 🌍 Often seen in children and young adults; can affect multiple organs.
⚙️ Aetiology
- 🦠 First human tumour proven to be associated with a virus: Epstein–Barr Virus (EBV).
- 🩸 Cancer of the lymphatic system with rapid doubling time.
- 🧾 Most cases linked to translocations involving the MYC gene on chromosome 8.
🧬 Translocations
- t(8;14)(q24;q32) – most common (70%).
- t(2;8)(p12;q24).
- t(8;22)(q24;q11).
📂 Types
- 🌍 Endemic: Equatorial Africa; EBV-associated; often affects the jaw/face in children (4–7 yrs).
- 🌐 Sporadic: More common worldwide; abdominal and bone marrow involvement, adolescents/young adults.
- 🧩 Immunodeficiency-related: Linked to HIV/AIDS, post-transplant; often EBV-negative.
🩺 Clinical Features
- 👶 Predominantly in children/young adults.
- 🦷 Endemic: jaw/face swelling, tooth displacement.
- 🌡️ Sporadic: abdominal masses, bowel obstruction, hepatosplenomegaly.
- 🧠 Possible CNS, kidney, ovary, or marrow involvement.
- 🩸 Leukaemic phase: resembles acute lymphoblastic leukaemia (L3 morphology).
🧠 Differentials
- Diffuse large B-cell lymphoma (DLBCL).
🔬 Investigations
- 📊 Bloods: FBC, U&E, LFTs, Ca, LDH (raised in bulky disease).
- 🧪 Excisional biopsy = gold standard (starry-sky histology).
- 🔎 Flow cytometry for immunophenotype confirmation.
- 🖥️ Imaging: CT CAP + PET for staging.
- 🧠 Consider lumbar puncture (CNS spread common).
💊 Management
- 🚨 Staging must be rapid – therapy within 48h of diagnosis.
- 🔪 Surgical resection if large obstructive abdominal mass.
- 💉 High-intensity, short-duration combination chemotherapy:
– CODOX-M/IVAC
– BFM regimens
– Rituximab often added.
- 💊 Supportive care: tumour lysis prophylaxis (allopurinol/rasburicase, hydration).
- 🧠 CNS prophylaxis with intrathecal methotrexate.
- 🧬 Stem cell transplantation: for relapse or refractory disease.
🧾 Case Vignettes
Case 1 – Endemic Burkitt’s 🦷
A 6-year-old boy from Uganda presents with a rapidly enlarging jaw mass and tooth loosening. Biopsy shows a starry-sky pattern and EBV positivity.
👉 Key point: endemic, EBV-driven, facial/jaw involvement.
👉 Management: urgent chemo ± rituximab, CNS prophylaxis.
Case 2 – Sporadic Burkitt’s 🌐
A 17-year-old male in the UK presents with acute abdominal pain and a palpable mass. CT shows ileocaecal mass with intussusception.
👉 Key point: sporadic type, GI involvement, emergency presentation.
👉 Management: surgery for obstruction, then intensive chemotherapy.
Case 3 – Immunodeficiency-associated Burkitt’s 🧩
A 32-year-old HIV-positive man with poor ART adherence presents with rapidly progressive cervical lymphadenopathy and night sweats. Biopsy confirms Burkitt lymphoma, EBV-negative.
👉 Key point: HIV-associated, aggressive course.
👉 Management: HAART optimisation + intensive chemo.
📚 References